Cochlear Implantation in a Patient with Neurofibromatosis Type 2 and an Intracochlear Schwannoma: A Case Report and Literature Review

作者：Dejun Zhang, Zeming Fu, Shuang Han, Fang Guo, Na Tang, Ying Zhao, Guofang Guan · 发表于：ORL · 年份：2022 · DOI：10.1159/000522473 · 被引用次数：4 · 研究领域：Meningioma and schwannoma management、Neurofibromatosis and Schwannoma Cases、Facial Nerve Paralysis Treatment and Research

A primary intracochlear schwannoma (ICS) is a unique type of vestibular schwannoma (VS); the tumor originates from the terminal branches of the cochlear nerve and is confined to the cochlea. An ICS is the most common subtype of schwannoma in the inner ear. As an ICS is clinically rare, diagnosis and treatment remain challenging. We report a rare case of cochlear implantation (CI) in a patient with neurofibromatosis type 2 and an ICS. The patient exhibited bilateral, profound, sensorineural hearing loss. The tumor on one side was a common VS treated via tumor and acoustic nerve resection and that on the other side an ICS. To ensure auditory rehabilitation via CI, we performed CI while removing part of the ICS via an enlarged round window. Auditory rehabilitation was satisfactory. Thus, ICS patients, especially those who urgently require auditory rehabilitation, can undergo simultaneous CI and (total or partial) tumor removal. However, the long-term results require close observation.